Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort

Background: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure.

Methods: All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both).

Results: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival.

Conclusions: Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.